Outcomes in persistent hyperplastic primary vitreous

AIMS: To report outcomes in cases of persistent hyperplastic primary vitreous (PHPV) and examine factors predictive of visual acuity in patients selected for surgery. METHODS: Retrospective case series of 55 eyes in 50 patients presenting between 1990 and 2001 at the Children's Hospital, Westmead. 33 eyes underwent surgery aimed at visual rehabilitation. RESULTS: In surgical patients, median age at surgery was 77 days, and median age at final follow up was 28 months. Six (18%) achieved a visual acuity at final follow up of 6/60 or better and eight (24%) achieved acuity of less than 6/60 to counting finger vision inclusive. Those undergoing surgery before 77 days were approximately 13 times more likely to obtain a visual acuity of counting fingers or better than those operated later (p = 0.01). Neither posterior segment PHPV nor post-surgical glaucoma was a significant predictor of outcome in this selected group. CONCLUSION: Early surgery aimed at rehabilitation markedly improves the likelihood of useful visual outcome in selected eyes with PHPV.     

Phacoanaphylactoid reaction in persistent hyperplastic primary vitreous

Three cases of persistent hyperplastic primary vitreous (PHPV) showing a ruptured lens capsule and a phacoanaphylactoid reaction are presented. In two cases, there was evidence that the lens had been clinically clear in the presence of a ruptured capsule and posterior cortical granulomatous reaction. The capsular rupture and granulomatous reaction can begin in utero, as shown by the third case, a premature who died at one day of age. It is suggested that a phacoanaphylactoid reaction can contribute to the lens swelling, cataract, and anterior chamber narrowing which often initiate the downhill course in eyes with PHPV. The immunological implications of this reaction are discussed.     

永存性原始玻璃体增生症的手术治疗

目的 观察永存原始玻璃体增生症(PHPV)的手术治疗效果。 方法 回顾性分析接受玻璃体视网膜手术治疗的16例PHPV患者16只眼的临床资料。患者中男性9例，女性7例，年龄3个月~25岁，平均年龄51.9个月。14例为单眼发病，2 例为双眼发病，均未合并全身异常。其中，前部型3只眼，混合型13只眼；10只眼合并白内 障，7只眼合并虹膜后粘连，5只眼合并浅前房，3只眼合并角膜带状变性，1只眼角膜混浊， 2只眼合并牵引性视网膜脱离，1只眼合并牵引性孔源性视网膜脱离。手术前视力1只眼为光感，1只眼为眼前手动，1只眼为数指/10 cm，1只眼为0.02，12只眼不能配合视力检查，对强光刺激反应不明显。13只眼行晶状体切除和前段玻璃体切割，1只眼1期植入人工晶状体， 3只合并视网膜脱离眼行晶状体切除、玻璃体切割、眼内激光光凝、注气、巩膜环扎手术 。 手术后随访时间6个月至4年，平均随访时间为15.3个月。 结果 所有接受手术治疗眼手术后眼压均正常，前房形成，视网膜复位。混合型者残留视盘前纤维血管膜及视网膜皱襞。2例患者经弱视训练后最佳矫正视力达02和01，8例不配合视力检查者有遮盖厌恶反应，均对强光刺激有反应，6例患者视力低于数指。 结论 对于前部型和混合型PHPV患者，早期晶状体切除及玻璃体手术可以重建视觉通道并解除牵引 ，保存视力，减少继发性青光眼等并发症的发生，结合手术后弱视训练可获得有用视力。 （中华眼底病杂志，2008，24：210-212）     

永存原始玻璃体增生症的临床分析

目的探讨永存原始玻璃体增生症(persistent hyperplastic primary vitreous,PHPV)经过手术或非手术治疗的视力预后及影响因素。方法回顾性分析我院2007年1月至2009年12月23例(25眼)PHPV患者的临床资料,20眼行玻璃体或晶状体切除手术,5眼未手术。术后随访1.0~2.5a,观察临床特征、视力预后及术后并发症等情况。结果 B超检查示25眼均有从视盘发出的蘑菇状回声;彩超示玻璃体内条索状回声影内有连续血流,由视盘向晶状体后延伸。手术组术后最佳矫正视力达有用视力14眼,其中小眼球畸形1眼,正常眼轴13眼,二者相比差异有统计学意义(P=0.014);视网膜脱离、皱襞及发育异常者3眼,无该病变者11眼,二者比较差异有统计学意义(P=0.018)。非手术组随访期内最佳矫正视力无一眼获得有用视力。术后手术组慢性低眼压、玻璃体出血1眼。结论 PHPV患者通过手术治疗可以改善视力、防止并发症;未手术治疗者视力预后差。视力预后与小眼球畸形、视网膜脱离、皱襞及发育异常有关。     

Surgical results of persistent hyperplastic primary vitreous

PurposeTo evaluate the surgical success of patients with persistent hyperplastic primary vitreous (PHPV) and to identify preoperative indicators of visual outcome.DesignNoncomparative case series.MethodsThe diagnosis of PHPV was made in 35 eyes of 27 patients from 1982 to 1994. In each case, anterior and/or posterior PHPV findings, preoperative testing, surgical procedures, and visual outcomes were documented. Twenty-nine of 35 eyes were managed surgically. Follow-up ranged from 2 months to 12 years.ResultsOf the 35 eyes, 2 (5.7%) had strictly anterior PHPV, 8 (22.9%) had strictly posterior PHPV, and 25 (71.4%) had components of both anterior and posterior disease. Initial lensectomy and vitrectomy was performed in 24 eyes (68.6%). Surgery was withheld in four eyes secondary to severity of disease with an unrecordable visual-evoked potential (VEP). Reoperation rate was 32.3% for membrane reproliferation, glaucoma, vitreous hemorrhage, retinal detachment, or strabismus. Best-corrected final visual acuity ranged from 20/60 to no light perception. Six eyes (17%) maintained Snellen visual acuity despite posterior PHPV with some degree of retinal dysplasia.ConclusionsSurgical treatment of PHPV can result in functional visual outcome despite posterior segment involvement. The degree of ocular malformation, however, will ultimately limit the amount of visual improvement. Preoperative testing, including VEP, may aid in determining surgical candidates.     

Approach to cataract with persistent hyperplastic primary vitreous

Persistent hyperplastic primary vitreous (PHPV) is a congenital anomaly in which the hyaloid vasculature persists beyond fetal life. In cases in which cataract is associated with PHPV, intraoperative bleeding is a potential complication during cataract surgery. The pars plana approach along with endocoagulation has been used in such cases. We describe an alternative approach using a Fugo plasma blade via an anterior route. This approach provides better control over the posterior capsulotomy along with minimal traction over the retina and, most important, hemostasis during surgery.     

Familial exudative vitreoretinopathy mimicking persistent hyperplastic primary vitreous

PURPOSE: To report an unusual case of familial exudative vitreoretinopathy in an infant. METHODS: Case report. A 6-day-old girl had unilateral microphthalmia in the right eye, with a retrolental plaque initially diagnosed as persistent hyperplastic primary vitreous. Three months later, peripheral retinal vascular changes and a fibrovascular ridge were noted in the left eye, suggesting familial exudative vitreoretinopathy as the cause in both eyes. RESULTS: The microphthalmic right eye was unsalvageable. The left eye developed an exudative retinal detachment despite photocoagulation of the peripheral avascular retina. Additional cryotherapy resulted in resolution of the detachment and regression of the vascular changes. CONCLUSIONS: With highly asymmetric involvement, neonatal familial exudative vitreoretinopathy can mimic persistent hyperplastic primary vitreous. Fellow eye involvement can progress rapidly.     

Neurologic abnormalities associated with persistent hyperplastic primary vitreous

BACKGROUND: Persistent hyperplastic primary vitreous (PHPV) is usually a unilateral ocular abnormality not associated with other systemic findings. We describe 16 patients, 7 with unilateral and 9 with bilateral PHPV, in whom significant neurologic abnormalities were identified. METHODS: The clinical records of 16 children referred to the Visually Impaired Program at a university-affiliated children's hospital in Vancouver were studied from 1970 to 1996. Further investigations, including electroencephalography, computed tomography and pediatric neurologic evaluation, were performed to ascertain neurologic deficits. RESULTS: Neurologic abnormalities were identified in three of the children with unilateral PHPV (hemiparesis, impaired coordination and homonymous hemianopia) and seven of the children with bilateral PHPV (ataxia, impaired coordination, hypotonia, spastic quadriplegia, microcephaly and deafness). INTERPRETATION: The possibility of underlying neurologic abnormality should be considered in patients with PHPV, particularly those with bilateral disease. Referral to a pediatric neurologist may be appropriate.     

Ultrastructural pathology of anterior persistent hyperplastic primary vitreous

Using transmission electron microscopy, the fine structure of anterior persistent hyperplastic primary vitreous (PHPV) removed from the eyes of four infants was studied. The tissue mass was composed of vessels derived from or representing the tunica vasculosa lentis posterior and the vasa hyaloidea propria. They were embedded in a loose connective-tissue matrix containing many fibroblasts and mature collagen fibrils. Toward the posterior surface, the fibroblasts became more numerous, elongated and densely packed. At the point of its entry into the posterior pole of the mass, the hyaloid artery was found to be surrounded by glial cells, probably representing an extension of the wall cells of the hyaloid canal. Venous drainage of the PHPV nodules seemed to occur via the ciliary body to which they were connected by tiny vessels bridging the distance from the PHPV lump to elongated ciliary processes. The anterior surface of the tissue lump was covered with lens fragments. These remnants showed signs of very early disturbance of lens development, with a failure to form posterior lens fibers. Based on these findings, the authors sugget that PHPV may be secondary to a primary defect in lens development.     

Locus for autosomal recessive nonsyndromic persistent hyperplastic primary vitreous

PURPOSE: To map the disease locus in a six-generation, consanguineous Pakistani family affected by nonsyndromic autosomal recessive persistent hyperplastic primary vitreous (arPHPV). All affected individuals had peripheral anterior synechiae and corneal opacities with variable degrees of cataract and a retrolenticular white mass behind the lens. METHODS: Genomic DNA from family members was typed for alleles at more than 400 known polymorphic genetic markers, by polymerase chain reaction. Alleles were assigned to individuals, which allowed calculation of lod scores. RESULTS: A maximum two-point lod score of 4.07 was obtained with marker D10S1225 with no recombination. Two recombinations with marker D10S208 and D10S537 localized the disease within a region of approximately 30 centimorgans (cM). However, homozygosity across the region refined the arPHPV locus to 13 cM. CONCLUSIONS: Linkage analysis shows localization of nonsyndromic arPHPV to chromosome10q11-q21.     

Unilateral persistent hyperplastic primary vitreous: course and outcome.

PURPOSE: Improved surgical techniques enable more favorable results in the management of persistent hyperplastic primary vitreous (PHPV). The purpose of our study was to evaluate the outcome of PHPV eyes managed conservatively or after surgery (with or without intraocular lens implantation). METHODS: A total of 89 children (37 boys, 52 girls) with PHPV in one eye (mean follow-up of 6.3 +/- 5.7 years) were included. The children were subgrouped according to treatment modality. Twenty-eight (31.5%) children were managed conservatively (nonoperated). Cataract extraction combined with vitrectomy and removal of embryonic remnants was carried out in 61 eyes (68.5%). Intraocular lenses were implanted in 30 of the operated eyes (pseudophakic) and 31 eyes remained without lens (aphakic). RESULTS: Final evaluated visual acuity in the entire group was 6/15 or better in 12.6% (11 of 87) of the eyes. A total of 11.5% (10 of 87) had a visual acuity of 6/21 to 6/60, 46.0% (40 of 87) obtained 6/90 to light perception and 26 of 87 (29.9%) had no light perception in the involved eye. The rate of no light perception was significantly lower in patients with pseudophakia (10.0%) compared to those with aphakia (43.3%) or nonoperated (37.0%) eyes (P =.009). Intraocular pressure was adequately assessed repeatedly in 72 eyes. High intraocular pressure and glaucomatous changes were observed in 7 of 31 (22.6%) patients with aphakia, 2 of 24(8.3%) patients with pseudophakia and in 2 of 17(11.8%) nonoperated eyes (P =.34). Poor cosmetic outcome was seen in 12 of 31 (38.7%) children with aphakic eyes and 5 of 30 (16.7%) children with pseudophakic eyes (P =.08). Prosthesis or cosmetic shells were needed for 8 of 31 patients with aphakia, for none of the patients with pseudophakia and for 2 of 28 of the nonoperated children (P =.003). CONCLUSION: PHPV eyes have a potential for developing useful vision with favorable cosmetic outcome after surgery. Intraocular lens implantation may be a favorable and beneficial option for the management of children with unilateral PHPV.     

Buphthalmos in the setting of persistent hyperplastic primary vitreous cataract

PURPOSE: To report a case of unilateral persistent hyperplastic primary vitreous cataract presenting with ipsilateral buphthalmos. DESIGN: Retrospective case report. METHODS: The patient chart was reviewed, and a relevant literature search was performed. RESULTS: A four-month-old child presented with persistent hyperplastic primary vitreous cataract in the right eye and ipsilateral buphthalmos with no discernable angle abnormalities. He underwent lensectomy, anterior vitrectomy, and limited transscleral diode cyclodestruction in the right eye. CONCLUSIONS: This appears to be the first report of buphthalmos presenting with unilateral persistent hyperplastic primary vitreous. Despite two comorbidities, the patient has thus far responded well to initial treatment and visual rehabilitation.